What are the management options for fibrosis?

Management Options for Pulmonary Fibrosis

Nintedanib and pirfenidone are the only approved antifibrotic medications for the treatment of idiopathic pulmonary fibrosis (IPF) and should be first-line therapy to reduce decline in pulmonary function and improve clinical outcomes. 1

Diagnosis and Assessment

• Diagnosis of IPF requires:

  • Exclusion of other known causes of interstitial lung disease
  • Abnormal pulmonary function tests showing restriction and/or gas exchange abnormality
  • Characteristic abnormalities on HRCT in patients with consistent clinical presentation 1

• Regular monitoring is essential:

  • Pulmonary function tests every 3-6 months
  • HRCT if unexplained clinical changes or suspected acute exacerbation
  • Assessment for disease progression markers: FVC decline ≥10% or DLCO decline ≥15% 2, 1

Pharmacological Management

Antifibrotic Medications

• First-line therapy options:

  • Nintedanib - 150 mg twice daily with regular assessment of FVC decline 1
  • Pirfenidone - with regular liver function tests and clinical assessment 1

• Important considerations:

  • Both medications slow disease progression but do not cure IPF
  • Regular monitoring for adverse effects is required
  • Pharmacologic treatment should be limited to carefully selected patients willing to accept possible adverse consequences 2

Medications to Avoid

• Corticosteroids should not be used routinely in IPF as they:
  • Have not been shown to improve survival
  • Are associated with substantial morbidity, especially in older patients 1
  • May be considered only for acute exacerbations (weak recommendation) 2

Non-Pharmacological Interventions

Pulmonary Rehabilitation

• Should be considered to improve exercise capacity and quality of life
• May not be feasible in patients with advanced disease 1

Oxygen Therapy

• Recommended for patients with:
  • Clinically significant resting hypoxemia
  • Exercise desaturation (below 88% during formal 6MWT) 2, 1
• Should be measured at rest and with exertion in all patients regardless of symptoms 2

Lung Transplantation

• Should be considered in all patients with definite IPF under age 65 if:
  • Disease is severe or worsening
  • Patient has increased risk of mortality 2, 1
• Early evaluation at a lung transplant center is recommended 1

Symptom Management

Cough Management

• Options include:
  • Trial of inhaled ipratropium
  • Gabapentin for refractory chronic cough
  • Speech pathology therapy 1

Dyspnea Management

• Opiates may be recommended for persistent, troublesome symptoms that impact quality of life
• Regular reassessment is needed (at 1 week and monthly thereafter) 1

Preventive Care

• Annual influenza and pneumococcal vaccinations
• Smoking cessation
• Psychosocial support 1

Monitoring for Complications

• Regular assessment for:

  • Pulmonary hypertension (especially in patients with progressive disease)
  • Lung cancer
  • Coronary artery disease
  • Pulmonary embolism 2

• Echocardiography is inaccurate in estimating pulmonary hemodynamics in fibrotic lung disease 2

Pitfalls and Caveats

1. Diagnostic delays are common and harmful - early diagnosis is crucial for timely treatment initiation 3

2. Overreliance on small changes in PFTs - isolated changes of <5% in FVC and <10% in DLCO should be interpreted with caution as they may overlap with test variability 2

3. Hepatotoxicity monitoring - both antifibrotic medications require regular liver function monitoring 1, 4

4. Mechanical ventilation is not recommended for the majority of patients with respiratory failure due to disease progression 2

5. Advanced directives must be discussed early in the ambulatory setting 2